Proteomics

Dataset Information

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Complexome profiling of brain mitochondrial membranes from Clpp-/- mouse

ABSTRACT: The autosomal recessive Perrault syndrome with juvenile ovarian failure, progressive sensorineural deafness, ataxia and leukoencephalopathy can be caused by loss of function mutations in CLPP. This gene encodes a peptidase that is conserved since bacteria and localizes to mitochondrial matrix in eukaryotes. Here, assembly and stability of mitochondrial complexes from adult Clpp-/- mouse brains were analyzed by Complexome profiling.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Brain

SUBMITTER: Ilka Wittig  

LAB HEAD: Georg Auburger

PROVIDER: PXD025478 | Pride | 2022-02-17

REPOSITORIES: Pride

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Publications

Increased presence of nuclear DNAJA3 and upregulation of cytosolic STAT1 and of nucleic acid sensors trigger innate immunity in the ClpP-null mouse.

Maletzko Antonia A   Key Jana J   Wittig Ilka I   Gispert Suzana S   Koepf Gabriele G   Canet-Pons Júlia J   Torres-Odio Sylvia S   West A Phillip AP   Auburger Georg G  

Neurogenetics 20210803 4

Mitochondrial dysfunction may activate innate immunity, e.g. upon abnormal handling of mitochondrial DNA in TFAM mutants or in altered mitophagy. Recent reports showed that also deletion of mitochondrial matrix peptidase ClpP in mice triggers transcriptional upregulation of inflammatory factors. Here, we studied ClpP-null mouse brain at two ages and mouse embryonal fibroblasts, to identify which signaling pathways are responsible, employing mass spectrometry, subcellular fractionation, immunoblo  ...[more]

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